Mitochondrial fatty acid biosynthesis and muscle fiber plasticity in very long‐chain acyl‐CoA dehydrogenase‐deficient mice

Tucci, Sara; Mingirulli, Nadja; Wehbe, Zeinab; Dumit, Verónica I.; Kirschner, Janbernd; Spiekerkoetter, Ute

doi:10.1002/1873-3468.12940

Cited by 14 publications

(8 citation statements)

References 56 publications

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“…Similar to the observations in humans, long‐term treatment studies VLCAD −/− mice demonstrated that triheptanoin was indeed able to supply enough energy at rest . In addition, the metabolic and morphologic trans‐differentiation towards the glycolytic muscle fibres type II observed as progressive adaptive mechanism was not reversed by the supplementation with this compound . On the other hand, at the cardiac level, although triheptanoin was not able to prevent the impairment of cardiac function, it did not contribute to progression of cardiac dysfunction in dilated cardiomyopathy in contrast to the regular MCT, similar to data reported for FAOD patients .…”

Section: Methodssupporting

confidence: 86%

“…47 In addition, the metabolic and morphologic trans-differentiation towards the glycolytic muscle fibres type II observed as progressive adaptive mechanism was not reversed by the supplementation with this compound. 48 On the other hand, at the cardiac level, although triheptanoin was not able to prevent the impairment of cardiac function, it did not contribute to progression of cardiac dysfunction in dilated cardiomyopathy in contrast to the regular MCT, similar to data reported for FAOD patients. 43,47,49 Moreover, long-term supplementation of triheptanoin induced de novo lipogenesis and elongation of fatty acids leading to the alteration of the fatty acid profiles in several tissues characterised by a strong reduction of polyunsaturated fatty acids and the marked increase of monounsaturated species.…”

Section: Triheptanoin In Long-chain Fatty Acid Disorderssupporting

confidence: 72%

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Therapeutic potential of triheptanoin in metabolic and neurodegenerative diseases

Wehbe

Tucci

2019

J of Inher Metab Disea

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In the past 15 years the potential of triheptanoin for the treatment of several human diseases in the area of clinical nutrition has grown considerably. Use of this triglyceride of the odd-chain fatty acid heptanoate has been proposed and applied for the treatment of several conditions in which the energy supply from citric acid cycle intermediates or fatty acid degradation are impaired.Neurological diseases due to disturbed glucose metabolism or metabolic diseases associated with impaired β-oxidation of long chain fatty acid may especially take advantage of alternative substrate sources offered by the secondary metabolites of triheptanoin. Epilepsy due to deficiency of the GLUT1 transporter, as well as diseases associated with dysregulation of neuronal signalling, have been treated with triheptanoin supplementation, and very recently the advantages of this oil in long-chain fatty acid oxidation disorders have been reported. The present review summarises the published literature on the metabolism of triheptanoin including clinical reports related to the use of triheptanoin. K E Y W O R D Sanaplerosis, epilepsy and neurodegenerative diseases, metabolic diseases, triheptanoin

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Section: Methodssupporting

confidence: 86%

Section: Triheptanoin In Long-chain Fatty Acid Disorderssupporting

confidence: 72%

Therapeutic potential of triheptanoin in metabolic and neurodegenerative diseases

Wehbe

Tucci

2019

J of Inher Metab Disea

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“…In addition, the glycolysis/gluconeogenesis, closely related to skeletal muscle fiber types, is significantly enriched. Moreover, fatty acid metabolism and fatty acid degradation pathways are also significantly enriched, while recent studies found that the change of fatty acid composition was accompanied by the transformation of skeletal muscle fiber types [40,41]. Thus, it will be interesting to investigate the specific regulatory mechanisms of the skeletal muscle fiber types and fatty acid composition mediated by the DE lncRNAs identified in this study.…”

Section: Discussionmentioning

confidence: 80%

Exploring the lncRNAs Related to Skeletal Muscle Fiber Types and Meat Quality Traits in Pigs

Jiang

et al. 2020

Genes

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The alteration in skeletal muscle fiber is a critical factor affecting livestock meat quality traits and human metabolic diseases. Long non-coding RNAs (lncRNAs) are a diverse class of non-coding RNAs with a length of more than 200 nucleotides. However, the mechanisms underlying the regulation of lncRNAs in skeletal muscle fibers remain elusive. To understand the genetic basis of lncRNA-regulated skeletal muscle fiber development, we performed a transcriptome analysis to identify the key lncRNAs affecting skeletal muscle fiber and meat quality traits on a pig model. We generated the lncRNA expression profiles of fast-twitch Biceps femoris (Bf) and slow-twitch Soleus (Sol) muscles and identified the differentially expressed (DE) lncRNAs using RNA-seq and performed bioinformatics analyses. This allowed us to identify 4581 lncRNA genes among six RNA libraries and 92 DE lncRNAs between Bf and Sol which are the key candidates for the conversion of skeletal muscle fiber types. Moreover, we detected the expression patterns of lncRNA MSTRG.42019 in different tissues and skeletal muscles of various development stages. In addition, we performed a correlation analyses between the expression of DE lncRNA MSTRG.42019 and meat quality traits. Notably, we found that DE lncRNA MSTRG.42019 was highly expressed in skeletal muscle and its expression was significantly higher in Sol than in Bf, with a positive correlation with the expression of Myosin heavy chain 7 (MYH7) (r = 0.6597, p = 0.0016) and a negative correlation with meat quality traits glycolytic potential (r = −0.5447, p = 0.0130), as well as drip loss (r = −0.5085, p = 0.0221). Moreover, we constructed the lncRNA MSTRG.42019–mRNAs regulatory network for a better understanding of a possible mechanism regulating skeletal muscle fiber formation. Our data provide the groundwork for studying the lncRNA regulatory mechanisms of skeletal muscle fiber conversion, and given the importance of skeletal muscle fiber types in muscle-related diseases, our data may provide insight into the treatment of muscular diseases in humans.

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“…Peptides were extracted from the gel slices with ethanol and resulting peptide mixtures were processed on STAGE tips as described [34]. Sample analysis, data acquisition and processing were performed as previously described [35].…”

Section: Methodsmentioning

confidence: 99%

Proteomic Phosphosite Analysis Identified Crucial NPM-ALK-Mediated NIPA Serine and Threonine Residues

Gengenbacher

Müller-Rudorf

Poggio

et al. 2019

IJMS

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Anaplastic large-cell lymphoma (ALCL) is an aggressive non-Hodgkin lymphoma that shows in 60% of cases a translocation t(2;5)(p23;q35), which leads to the expression of the oncogenic kinase NPM-ALK. The nuclear interaction partner of ALK (NIPA) defines an E3-SCF ligase that contributes to the timing of mitotic entry. It has been shown that co-expression of NIPA and NPM-ALK results in constitutive NIPA phosphorylation. By mass spectrometry-based proteomics we identified nine serine/threonine residues to be significantly upregulated in NIPA upon NPM-ALK expression. Generation of phospho-deficient mutants of the respective phospho-residues specified five serine/threonine residues (Ser-338, Ser-344, Ser-370, Ser-381 and Thr-387) as key phosphorylation sites involved in NPM-ALK-directed phosphorylation of NIPA. Analysis of the biological impact of NIPA phosphorylation by NPM-ALK demonstrated that the ALK-induced phosphorylation does not change the SCFNIPA-complex formation but may influence the localization of NIPA and NPM-ALK. Biochemical analyses with phospho-deficient mutants elucidated the importance of NIPA phosphorylation by NPM-ALK for the interaction of the two proteins and proliferation potential of respective cells: Silencing of the five crucial NIPA serine/threonine residues led to a highly enhanced NIPA-NPM-ALK binding capacity as well as a slightly reduced proliferation in Ba/F3 cells.

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Mitochondrial fatty acid biosynthesis and muscle fiber plasticity in very long‐chain acyl‐CoA dehydrogenase‐deficient mice

Cited by 14 publications

References 56 publications

Therapeutic potential of triheptanoin in metabolic and neurodegenerative diseases

Therapeutic potential of triheptanoin in metabolic and neurodegenerative diseases

Exploring the lncRNAs Related to Skeletal Muscle Fiber Types and Meat Quality Traits in Pigs

Proteomic Phosphosite Analysis Identified Crucial NPM-ALK-Mediated NIPA Serine and Threonine Residues

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